Subgroups of primary Sjögren's syndrome. Sjögren's syndrome in male and paediatric Greek patients.

OBJECTIVES: To describe the clinical and serological findings in male and paediatric Sjögren's syndrome (SS) patients. PATIENTS AND METHODS: Using the European criteria for the diagnosis of SS 12 male and 13 paediatric patients were identified and compared with those of 30 consecutive unselected adult female SS patients. RESULTS: The mean (SD) age of paediatric patients was 9.4 (2.2) years, ranging from 6 to 14 years. Recurrent parotid gland enlargement was the initial clinical manifestation in the majority of the children with a statistical significance compared with male (p < 0.01) and with female patients (p < 0.0001). Sicca manifestations were the most common clinical symptoms in male and female patients at disease onset. The systemic manifestations were similar among the three groups except that men showed lower frequency of arthritis (p < 0.05) and Raynaud's phenomenon (p < 0.05) compared with women. No differences were found among the immunological profile of children and female patients, while male patients had a lower frequency of antinuclear antibodies (p < 0.025) and antibodies to Ro(SSA) nuclear antigens (p < 0.025) compared with women. CONCLUSION: Primary SS is rare in children and men in Greece. Recurrent parotid gland enlargement is the most common clinical finding at disease onset in children. Male patients seem to have less systemic manifestations and lower frequency of autoantibodies.

Influence of methotrexate on radiographic progression in rheumatoid arthritis: a sixty-month prospective study.

OBJECTIVE: To evaluate if methotrexate (MTX) can slow disease progression, as determined radiographically, in comparison to other disease modifying drugs in rheumatoid arthritis (RA) patients. MATERIALS AND METHODS: Pairs of hand and wrist radiographs from 30 patients treated with MTX and 30 treated with D-penicillamine (DP) were evaluated blindly and separately by two radiologists (A and B) using reference radiographs for scoring. A scale scoring similar to Larsen's standard radiographs with minor modifications was used. The radiographs studied were obtained at the beginning and 5 years after therapy in both groups. RESULTS: A significantly greater worsening was found in the DP-treated patients (p = 0.025), as compared to MTX patients. Methotrexate showed a slower rate of disease progression than DP. Furthermore, in the MTX group 15 patients remained radiographically stable, 9 worsened and 6 were healed. In contrast, in the DP group 22 patients remained radiographically stable, 8 worsened and none improved. CONCLUSIONS: The rate of radiographic progression in RA patients was lower in MTX-treated patients compared to those treated with DP. Six patients showed radiological improvement after MTX treatment. Therefore, it seems that MTX could be considered a disease modifying drug.

D-penicillamine induced myasthenia gravis: clinical, serological and genetic findings.

Eight cases of D-penicillamine (DP) induced myasthenia gravis (MG) are presented. Seven patients were being treated for rheumatoid arthritis (RA) and one for scleroderma. The mean duration of DP treatment until the myasthenic symptoms developed ranged from 2-8 months. The DP dose reached 500 mg daily. It was found that the clinical and immunological findings were almost similar to those of idiopathic MG, but were less severe. All patients had increased titers of acetylcholine receptor antibodies in their sera. Discontinuation of D-penicillamine resulted in the complete resolution of myasthenic symptoms after 2-6 months. One patient required ventilation, immunosuppressive therapy and plasma exchange. No association was found between DP related MG and the various autoantibodies tested. Immunogenetic analysis showed that three patients had HLA-DR1, two HLA-DR3, one HLA-DR4 and one HLA-DR5. In conclusion, the clinical presentation of DP-induced MG seems similar to idiopathic MG. DP-related MG is relatively benign, although it sometimes can cause life-threatening muscle weakness requiring aggressive therapy. The relatively small number of patients included in this study, however, does not permit any firm conclusions regarding the HLA associations of DP-related MG.